Sickle Cell Disease, Animation
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- Опубликовано: 16 сен 2019
- (USMLE topics, cardiology, blood disorders) Genetics, different forms of SCD, pathophysiology, and treatment.
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Sickle cell disease is a group of inherited blood disorders in which the body produces abnormally-shaped red blood cells that look like crescent moons or sickles. Sickle cells have a shorter-than-normal life span; their premature destruction results in shortage of red cells, known as anemia. Signs of anemia include shortness of breath, fatigue, and delayed growth in children. Unlike normal red cells which are pliant, sickle cells are rigid and also sticky. They may clump together and stick to the walls of blood vessels, causing obstruction in small vessels and subsequent reduced oxygen supply to various organs. This happens repeatedly and manifests as periodic episodes of pain, called crises, which can last hours to days, and may result in organ damage, especially in the eyes, lungs, kidneys, bones and brain. The spleen has to handle large numbers of dead red cells and becomes enlarged and fibrous, its immune function declines, making the body more vulnerable to infections. In an attempt to compensate for blood cell loss, the bone marrow tries to produce more cells and grows larger, causing bones to weaken. Other signs include jaundice, a result of rapid destruction of heme.
Hemoglobin is the major component of red blood cells and is responsible for oxygen transport. The adult hemoglobin, or hemoglobin A, is composed of 4 protein chains: 2 alpha and 2 beta. The beta subunit is encoded by the HBB gene. Several mutations in HBB gene are responsible for the disease. Each individual has two copies of HBB gene. The disease develops when both copies are mutated, producing no normal beta globin. The 2 copies may be mutated differently, producing two different forms of abnormal beta subunits in the same person. Various combinations of these mutations produce different forms of sickle cell disease, but the most common and also most severe, called sickle cell anemia, is caused by 2 copies of the same mutation producing the mutated hemoglobin S. Each copy comes from a parent. The 2 parents each carry one copy of the mutated gene, but they typically do not show any symptoms. This pattern of inheritance is called autosomal recessive.
Hemoglobin S has the tendency to form polymers under low oxygen conditions. This process is called sickling, or gelation, for the gel-like consistency of the resulting polymer. As the polymer filaments grow, they eventually involve the cell membrane and distort the cell into the characteristic crescent shape. Apart from oxygen tension, the presence of other hemoglobins also seems to affect the sickling process. Normal adult hemoglobin inhibits sickling and this explains why heterozygous parents, who produce both mutated hemoglobin S and normal hemoglobin A, do not usually develop the disease. Fetal hemoglobin F, which has 2 gamma chains in place of 2 beta chains, also suppresses sickling. Infants born with the condition seem to benefit from high levels of fetal hemoglobin in the first few months of life: they do not develop symptoms until the age of 6 months or so, when fetal hemoglobin levels drop.
Bone marrow transplantation is currently the only known cure for sickle cell disease. It involves replacing the diseased stem cells in the bone marrow with healthy cells from an eligible donor, usually a relative. The procedure however is complex and finding a suitable donor can be difficult. In most cases, treatments aim to avoid crises, relieve symptoms and prevent complications.
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WOW every second is useful...no useless ads no useless self advertisement ..awesome mam
Great knowledge donors, indeed your explanations are very clear and comprehesive ,thank you for the quality services you are providing to us .
This is one of the detailed videos on Sickle Cell. Bravo!
I love that - clear, complete knowledge.
Your video helps us alot. Thank you. Easier and fun to study the subject now😊
All videos of this channel are very helpful and simple with detailed information ❤️❤️..
Please excuse my language, but F#%k this horrible disease. My older brother just passed away two nights again after battling sickle cell for 44 yrs. He was 6 weeks from his 45th bday. My only brother, I feel completely broken inside
I can feel yr pain I lost my only younger brother 3 years ago, please take it easy
Sorry for your loss , 😢
I feel so bad for you :( I have this disease aswell and it is indeed horrible everytime I feel pain it feel like I’m going to die #fucksicklecell
@@YoFrost
Do you have spleen problem?
My condolences,
But that means you're probably a carrier if you don't have it please find yourself a suitable partner for matting who is not a carrier.
You likely won't have maleria tbh props to that
As an EMT, this really helps. They told us to keep studying.
❤️
Thank you for the explanation
Thanks for this detail video 😊
amazing explanation. thank u very much
This vedio help me a lot in understanding the disease sickle cells anemia thinks
Wonderful thank you
Superb video, thanks
Thank you
Your all video is knowledgel madam I watch your all video
Thank you alot
So helpful.
thank you for this video
Very educative
A nice article.
Wonderful
Explaination
Thanku
My Pray Is May Our Doctors Find A Lasting Solution To This Challenge
Thank u
Excellent.
perfect
Thanku 😊😊😊
This is more accurate than Cells at Work. In the cartoon, they depicted the protagonist as a cell who goes back and forth, and can get in the capillaries fine.
🔥🔥🔥
Do you guys know where I can do my genotype in Munich
It should be possible in any hospital with a Haematolgy department. The test is called Haemoglobin Electrophoresis. You should ask your Hausarzt about it and he/she will tell you where you can do it near you
How do u differentiate between genetic disease anemia and nutritional deficient anemia??? Also any relationship between hbS and g6pd
The initial CBC helps with orientation , for example if u find that u have non regenerative microcytic hypochromic anemia it's mostly likely an iron deficiency anemia ( confirm with iron and ferritin levels)
In this case with sickle disease the anemia is normocytic/ normochromic and it's very regenerative ++ (reticulocytes are over 120.000)
Different factors help as well such as patient history and clinical presentations ++ ( for example if someone has a history of a gastric ulcer / heavy periods -> iron deficiency)
I’m in a very serious pain .. I feel like committing sucide right now.. this pain caused me to search this video. Goddddddddd
Please don't give up, get yourself to your doctor ASAP 🙏🏻 ❤ xxxx
Bamigboye Damilola , my neighbor has the worst kind of sickle cell anemia called SS .
I hope that in your lifetime they will find an all natural cure for this rotten illness .
I guess you are also suffering from hemochromatosis ?
He has it from not being able to get rid of iron and it fills up in his body and blood as well as other heavy metals .
My friend if you Google all natural things that help chelate or flush out iron you will see there are several of them such as quercetin also turmeric .
If you do the turmeric it must absolutely must have fresh cracked black pepper in it because the body cannot easily process the healing curcumin from turmeric .
So you want to make golden milk and on the NYISHAR channel he shows you the proper way to make it .
The fresh cracked black pepper makes the healing curcumin up to two thousand times more bioavailable and if you don't add it your body will not be able to get much of the curcumin which it needs .
Quercetin is also supposed to help .
You might want to also look into liquid is zeolite which is supposed to also flush out metals .
I wish you well ,my friend , I am so sorry that you're suffering .
Bamugboye , and that video He suggests good-quality sesame oil for the golden milk but please do not do that as it is high in copper and zinc which is the last thing you need .
I suggest you use GHEE which is a healthy fat and very good for inflammation and it is also high in calcium .
A LOT of CALCIUM helps BLOCK THE ABSORPTION of IRON .... you need to block the absorption of iron and other metals which caused the hemochromatosis in sickle cell anemia .
Please also be very careful of any dairy products , such as yogurt , and especially BAKED GOODS ... especially if you live in America ... because they often have IRON ADDED to them and that is the LAST THING you need .
So please do make sure you read the ingredients on everything you buy .
I feel sorry for you. I'm in a lot of pain right now and I want to kill myself too! This illness is so unfortunate... I can't stand it any longer!
Ask God our father for help, please believe in Jesus and god may help you.
This shit actually sucks 😔 my spleen is removed now im just a walking infection waiting to croak over and die due to my disease i was born with since birth 😮😢 i had to basically live n deal with these complications
That's me Erica Francisco
Wonderful
00:29
❤❤
I pray 🙏 there is a curative therapy
My neighbor has the worst form of this sickle cell anemia SS .
A specialist from California flies out two here in Dallas Texas to see him once a month .
One of the main problems with sickle cell anemia is that the person cannot get rid of iron and other heavy metals in their body ... they get hemochromatosis .
If you Google about that you will see there are certain all natural things you can take which helped chelate and flush out the iron and other heavy metals from the body ... for example quercetin or turmeric .
Liquid zeolite might help also .
I hope more people will look into these all natural Therapies
Added something
I have that and ima be doing a surgery soon they said ima have to stay 3 months in the hospital and its risky they said i wont be in the hospital all the time they gonna get me a hotel
Helpayi enn thoniyavar like adi
Yeh malayali 😉
Can someone use iron tablets for this.
Or only the Folic acid.
Thank you.
Actually the baby who has homozygous gene of sickle cell is lethal
Which leads to death of premature baby
Folic acid is more suitable in sickle cell than Iron
@@ndumferdy2545 hi i have 2 disease
1
G6pd
2 sickle cell
I'm falisha I hate that I'm so and I was born with it and it hurts my legs so much
I have it as well. I get hospitalized once a year for pain crisis in my arms and legs. We are sickle cell warriors
@@angelicfaithh2664 hi am sorry to hear u hve it too that sick make us really sick bad. Nd they don't have cure for it
Cora Lee ... some of the foods high in calcium of course are dairy products but if they are processed such as yogurt you need to read the ingredients to make sure they didn't add iron .
Many baked goods such as breads , cakes , and cookies all have iron added ... so again , please make sure you read the ingredients .
Breed it out?
On Jog , Well , with all genetic diseases , I'm sure they're working on it but ... time will tell ...
In Marathi hbs माहिती देण्यात यावी
50
I have sickle cell
I have hb s
Swati Sucharita ... do you mean you have the hemochromatosis ... and your body doesn't get rid of the iron and it builds up ?
Is so my neighbor also has that with the sickle cell anemia SS
Pretty certain it's this got worse so I no it you final stages
:30
Fun fact: I have this disease
Anger DF I feel so bad for you I know it's a really horrible disease .
My neighbor , Mark , has the worst form of this : sickle-cell SS .
We're here in Dallas Texas and once a month of research Doctor flies out from California here to treat him .
He gets the hemochromatosis from not being able to get rid of iron and other heavy metals from his body .
... I guess that's what happens to you , also ?
There are some all natural things which can help get rid of some of the iron and other metals in the body for example turmeric or quercetin .
You can look that up just look up things that flush out iron or things that flush out heavy metals from the body .
The turmeric won't work well if you don't prepare it properly ... the body cannot easily absorbed the healing curcumin out of the turmeric unless you use fresh grated pepper from the peppercorns which makes the curcumin 1 to 2,000 X to more bioavailable !
Probably best to prepare it as golden milk .
There is a great video on the NYISHAR channel that shows you how to make the golden milk .
When he says to add a good healthy fat such as sesame oil , he doesn't mean the kind you put in Chinese food , which is toasted ... but raw organic sesame oil which anyone can get at most Indian Grocers and it is very good for you .
My friend , if you don't already do these things , please look into it .
I don't like to see anyone suffer .
I hope you will be well and that in your lifetime there will be an all natural cure .
Really sorry that you have to go through this with that Dreadful disease .
Anger DF ... I came back here cuz I made a mistake big-time and I apologize .
If you make the golden milk whatever you do please do not use sesame oil take cuz I just remembered it has pretty high amounts of copper and zinc in it which you definitely don't want if you have the hemochromatosis from sickle cell anemia .
A good healthy fat to put in it is GHEE .
It also has a fair amount of calcium in it and you want to take high amounts of calcium if you can because they help block the absorption of iron .
If you live in America there is a problem with iron because they put it in so many processed foods especially baked goods like breads , cakes , cookies .
So please read the ingredients on everything that you buy that is either processed or pre-packaged to make sure you're not getting a bunch of iron .
You even have to be careful with prepared dairy products such as yogurt because some of those I think have iron added so once again please make sure you read the ingredients on everything you buy from now on .
I wish you well my friend
@@gardensofthegods what are you apologizing for
@@gardensofthegods thanks brother,
From Iraq 🤍
I can't walk
maltlevol 12 would help but they banned it they now call it snake oil
I have this
Oh gosh hw u headup wit this sick it make ppl sick bad
Destroyer Nicky , I feel really bad for you .
My neighbor has this ... and he has the worst kind : Sickle-cell Anemia SS which is very rare .